People with PI Have Problems with Their Immune System and Are More Likely to Get Infections

What is PI?

Primary immunodeficiency diseases (PI) are a group of rare, chronic disorders that affect the immune system.
The immune system helps the body fight infection.

How the Immune System Works

Diagram showing how part of the immune system responds to a threat like a virus by making antibodies that attach to the virus and tell other parts of the immune system to destroy the virus.

In people with PI, one or more key parts of the immune system is missing or does not work the way it should.
As a result, the immune system is less able to fight off infections.

What are the signs and symptoms of PI?

Everyone gets an infection occasionally (for example, cold, stomach flu, or infected cut).
But people with PI often have infections that are:
- Severe (requiring hospitalization or intravenous antibiotics)
- Persistent (hard to cure)
- Unusual (caused by a bacteria or virus that is not common)
- Recurrent (keep coming back, even with treatment)

10 Warning Signs of PI

Four or more ear infections within 1 year
Two or more serious sinus infections within 1 year
Two or more months on antibiotics with little effect
Two or more pneumonias within 1 year
Failure of infant to gain weight or grow normally
Recurrent deep skin or organ abscesses
Persistent thrush in mouth or fungal infection on skin
Need for intravenous antibiotics to clear infections
Two or more serious infections including blood infections
A family history of PI

What causes PI?

PI is caused by one or more genetic errors.
In many cases, these errors are hereditary (run in families).
However, not everyone diagnosed with PI has a family history of PI.
Different genetic errors can cause different types of PI. More than 400 different PI disorders have been identified.

Who gets PI?

PI can affect anyone of any race. Some types of PI affect the immune system from birth or early childhood. Other types may not cause problems until later in life.
About 1 in every 1,200 to 2,000 people has some form of PI.

How is PI treated?

PI cannot be cured, but most types of PI can be treated and managed.
The goal of treatment is to reduce the severity and number of infections.
One option is to replace missing or defective antibodies (immune globulins or Ig).
- Ig products are made by purifying blood plasma donated by people without PI.
- People with PI get regular treatments with these products to help keep their immune system strong.

Learn more about GAMMAKED for PI

Important Safety Information

What is GAMMAKED?

GAMMAKED^™ [Immune Globulin Injection (Human) 10% Caprylate/Chromatography Purified] is an immune globulin injection that is used to treat primary humoral immunodeficiency (PI) in patients 2 years of age and older, idiopathic thrombocytopenic purpura (ITP) in adults and children, and chronic inflammatory demyelinating polyneuropathy (CIDP) in adults.

What is the most important information I should know about GAMMAKED?

GAMMAKED should be infused under your skin (in the subcutaneous tissue). DO NOT inject GAMMAKED into a blood vessel or directly into a muscle.

GAMMAKED May Cause

Blood Clots (Thrombosis). Blood clots may occur in patients taking immune globulin intravenous (IGIV) products, including GAMMAKED. You may be at greater risk for blood clots if you are of advanced age, sit or lie for long periods, have a clotting condition or a history of blood clots, take estrogen hormones, have a central catheter, have thick blood, and/or if you have other conditions that put you at risk for cardiovascular disease. Blood clots may occur even if you do not have any of these known risk factors.

Impaired kidney function or kidney failure. IGIV products, particularly those that contain sugar (sucrose), have been reported to be associated with kidney dysfunction and damage, kidney failure, and death. Kidney damage and kidney failure happen more often in patients receiving IGIV products containing sucrose. GAMMAKED does not contain sucrose. You may be at greater risk for kidney failure if you have kidney disease, diabetes, are over age 65, are seriously dehydrated, have a blood infection (sepsis), have a blood condition called paraproteinemia, or take drugs that can damage your kidneys.

CONTRAINDICATIONS

Do not use GAMMAKED if:

You have a history of severe allergic reactions to human immune globulin. Tell your healthcare provider if you have had a serious reaction to other medicines that contain human immune globulin. Ask if you are not sure.
You have an immunoglobulin A (IgA) deficiency and have antibodies to IgA and have a history of allergic reactions. Tell your healthcare provider if you have an IgA deficiency or ask if you are not sure.

WARNINGS AND PRECAUTIONS

Severe allergic reactions may occur with IGIV products, including GAMMAKED. IgA deficient patients who have antibodies against IgA are at greater risk of developing severe allergic reactions. Your healthcare provider should have medications, such as epinephrine, to immediately treat any sudden severe allergic reactions.

If you are receiving GAMMAKED, you could experience higher than normal levels of protein in your blood, thick blood, or low sodium (salt) in your blood. This may prevent your blood from flowing easily and possibly lead to blood clots.

Brain inflammation or brain swelling called Aseptic Meningitis Syndrome (AMS) may occur infrequently with IGIV products, including GAMMAKED, especially if you receive a high dose or a rapid infusion.

Blood damage called hemolysis and hemolytic anemia can develop after treatment with GAMMAKED. Your healthcare provider will closely monitor you for signs and symptoms of hemolysis and hemolytic anemia.

Swelling of the lungs may occur in patients following IGIV treatment, including GAMMAKED. Your healthcare provider will monitor you for signs of lung damage (also known as transfusion-related acute lung injury [TRALI]).

GAMMAKED is made from human blood and, therefore, carries a risk of transmitting infectious agents, such as viruses, the agent of the variant Creutzfeldt-Jakob disease (vCJD), or unknown infectious agents. You should consult with your healthcare provider if you have any questions or concerns.

Be sure to tell your healthcare provider about your recent history of vaccinations. Live vaccines for diseases like measles, mumps, rubella and varicella may not work as well for you while you are receiving GAMMAKED. Tell your healthcare provider that you are taking GAMMAKED before you receive any vaccination.

ADVERSE REACTIONS

In clinical studies, the most common side effects of GAMMAKED were:

Increased cough, stuffy nose, sore throat, headache, asthma, nausea, fever, diarrhea, and sinus infection, when administered intravenously to patients with PI.
Swelling and itching at the injection site, fatigue, headache, upper respiratory infection, joint pain, diarrhea, nausea, sinus infection, bronchitis, depression, allergic skin reactions, redness of the skin, migraine, muscle pain, viral infection, and fever, when administered subcutaneously to patients with PI.
Headache, bruising, vomiting, fever, nausea, rash, abdominal pain, back pain, and indigestion in patients with ITP.
Headache, fever, increased blood pressure, chills, rash, nausea, joint pain, and weakness in patients with CIDP.

During treatment with GAMMAKED, be sure to tell your healthcare provider about any unusual symptoms you experience as they may indicate a possible side effect.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/MedWatch, or call 1-800-FDA-1088.

Please click here for the Full Prescribing Information for more information including the Boxed Warning, a description of who should not take GAMMAKED, and dosing and administration information.